- It is not truly a congenital disorder
- Often called infantile hypertrophic pyloric stenosis
- Results in hypertrophy and hyperplasia of pyloric sphincter in
neonatal period
- Mainly affects circular muscle fibres of pylorus
- Pylorus becomes elongated and thickened
- ? Due to failure of nitric oxide synthesis
- Results in gastric outflow obstruction, vomiting and dehydration
- Affects 3 per 1000 live births
- Male : female 4:1
- Most common in first born males
- Multifactorial inheritance
- Strong genetic factor
- Risk to son if affected mother = 20%
- Risk to daughter if affected mother = 7%
- Risk to son if affected father = 5%
- Risk to daughter if affected father = 2%
Clinical features
- Usually presents between 3 and 6 weeks of age
- Late presentation up to 6 months can occur
- Rapidly progressive projectile vomiting without bile
- Child hungry and often feeds immediately after vomiting
- Dehydration and alkalosis is a prominent clinical feature
- Clinical features of dehydration include:
- Sunken eyes
- Depressed anterior fontanelle
- Reduced skin turgor
- Dry mucous membranes
- Increased capillary refill time
- Lethargy
- Palpable 'tumour' in right upper quadrant best felt from left during
test feed
- Visible peristalsis often seen
- Diagnosis can be confirmed by abdominal ultrasound
- Needs assessment of length, diameter and thickness of the pylorus
- A wall thickness of great than 3mm supports the diagnosis
- Biochemically a hypochloraemic alkalosis exists
- Serum electrolytes and capillary gases should be measured
- They should be corrected prior to surgery
Picture provided by Fahid Abu-Zant, Neblus Speciality
Hospital, Neblus, Palestine
Treatment
- Correct dehydration over a 24 - 72 hour period
- Nasogastric tube is often required
- Ramstedt's pyloromyotomy first described in 1911
- Transverse right upper quadrant or circumumbilical incision
- Longitudinal incision in pylorus down to mucosa
- Incision extend from duodenum onto the gastric antrum
- Need to try and avoid mucosal perforation
- Feeding re-established within 12-24 hours of surgery
- Recurrence rarely occurs
- Complications are rare and mortality is negligible
- Persistent postoperative vomiting may be due to
- Delayed return of normal gastric motility
- Gastro-oesophageal reflux
- Inadequate pyloromyotomy
- Operation has been described using a laparoscopic approach
- No clear benefit has been demonstrated over a circumumbilical
approach
Bibliography
Hall N J, van der Zee J, Tan H L et al. Meta-analysis of
laparoscopic versus open pyloromyotomy. Ann Surg 2004;
240: 774-778.
Hernanz-Schulman M. Infantile hypertrophic pyloric stenosis.
Radiology 2003; 227: 319-331
Huddart S,
Bianchi A,
Kumar V,
Gough D C S.
Ramstedt's pyloromyotomy: circumumbilical versus transverse
approach.
Pediatr Surg Int 1993;
8:
395-396 |